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Mortality in sickle cell disease. Life expectancy and risk factors for early death.
Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death. Expand
Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes.
Hemolysis plays less of a role in the vaso-occlusive-viscosity complications of disease like the acute painful episode, osteonecrosis of bone and the acute chest syndrome, and agents that decrease hemolysis or restore NO bioavailability or responsiveness may have potential to reduce the incidence and severity of the hemolytic subphenotypes of sickle cell disease. Expand
Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.
Adult patients taking hydroxyurea for frequent painful sickle cell episodes appear to have reduced mortality after 9 of years follow-up, and indications for hydroxyUREa treatment should be expanded. Expand
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms
Csp12-L attenuates the inflammatory and innate immune response to endotoxins and in doing so may constitute a risk factor for developing sepsis. Expand
Predicting clinical severity in sickle cell anaemia
- M. Steinberg
- Biology, Medicine
- British journal of haematology
- 1 May 2005
The ability to predict the phenotype of an individual with sickle cell anaemia would allow a reliable prognosis and could guide therapeutic decision making and may eventually provide useful methods of foretelling the likelihood of certain complications. Expand
Spontaneous oxygen radical generation by sickle erythrocytes.
- R. Hebbel, J. Eaton, M. Balasingam, M. Steinberg
- Medicine, Chemistry
- The Journal of clinical investigation
- 1 December 1982
Data suggest that an excessive accumulation of oxidant damage in sickle erythrocyte membranes might contribute to the accelerated membrane senescence of these cells, and indicate that accumulation of oxidation could be a determinant of normal ery Throcyte membraneSenescence. Expand
Management of Sickle Cell Disease
- M. Steinberg
- 1 April 1999
One of every 600 black people in the United States has sickle cell anemia. In addition, sickle cell–hemoglobin C disease and sickle cell–β-thalassemia, which are other common genotypes of sickle ce...
Fetal hemoglobin in sickle cell anemia.
New efforts to induce high HbF levels in sickle cell disease beyond those achievable with the current limited repertory of Hbf inducers are spurred on. Expand
Genetic Signatures of Exceptional Longevity in Humans
In-silico analysis suggests that 90% of centenarians can be grouped into clusters characterized by different “genetic signatures” of varying predictive values for exceptional longevity. Expand
The paradox of hemoglobin SC disease.
The HbC gene enhances, by dehydrating the SC red cell, the pathogenic properties of HbS, resulting in a clinically significant disorder, but somewhat milder that sickle cell anemia (SCA). Expand